Defective translation initiation causes vanishing of cerebral white matter

Gert C. Scheper, Christopher G. Proud, Marjo S. van der Knaap

Research output: Contribution to journalReview articlepeer-review

31 Citations (Scopus)


Leukoencephalopathy with vanishing white matter (VWM) is one of the most prevalent inherited white-matter disorders, especially in Caucasian populations. VWM is unusual because of its sensitivity to febrile infections and minor head trauma. The basic defect of this enigmatic brain disease resides in the regulation of initiation of protein synthesis. Recently, undue activation of the unfolded-protein response has emerged as an important factor in the pathophysiology of VWM. Here, we discuss the mechanisms that might be responsible for the selective involvement of the brain white matter in VWM. At present, VWM research is in need of an animal model to study disease mechanisms and therapeutic interventions.

Original languageEnglish
Pages (from-to)159-166
Number of pages8
JournalTrends in Molecular Medicine
Issue number4
Publication statusPublished or Issued - Apr 2006

ASJC Scopus subject areas

  • Molecular Medicine
  • Molecular Biology

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